摘要目的 总结分析脱屑性间质性肺炎(desquamative interstitial pneumonia,DIP)患者的临床特点、诊治及预后,提高临床诊治水平.方法 报道北京医院收治的1例经病理诊断的DIP患者,回顾性分析国内外文献.以“desquamative interstitial pneumonia"为检索词,在PubMed数据库检索,检索时间截止至2015年12月,共检索到DIP相关英文文献共110篇,报道国外病例290例.以“脱屑性间质性肺炎”为检索词,分别在万方数据库和中国同方期刊数据库检索,检索时间截止至2015年12月,分别检索到相关中文文献57和20篇,报道患者38和60例.经阅读除外资料不详者及无病理诊断结果的文献,最终筛选获得65例,联合本例,针对66例进行文献复习.其中男44例(69.7％),女22例(33.3％),年龄0～67岁,平均年龄(33±22)岁,中位年龄34.5岁.比较吸烟相关性及非吸烟相关性DIP患者的临床表现差异.结果 患者男,55岁,表现为咳嗽、活动后气短,低氧血症.既往大量吸烟史及多种金属接触史.胸部CT示双肺磨玻璃影,行胸腔镜辅助下右肺上叶和中叶病变局部切除术,术后病理诊断脱屑性间质性肺炎.给予糖皮质激素治疗后好转.吸烟组20例,非吸烟者46例.吸烟组平均年龄(47.5±12.1)岁,与非吸烟组相比较高[(26.1±22.5)岁];吸烟组患者出现胸痛、气短症状(4/20、8/20)多于非吸烟组患者(0/46、3/46),胸部影像学表现以磨玻璃影为主(15/20),肺功能表现为弥散功能障碍(12/14).结论 脱屑性间质性肺炎是一种少见病,除吸烟外与多种危险因素相关.非吸烟导致DIP患者表现不典型.治疗对糖皮质激素敏感,预后良好,有复发可能.

abstractsObjective To analyze the clinical features,diagnosis,and treatment of desquamative interstitial pneumonia(DIP).Methods The clinical manifestation,radiology,and pathology were analyzed in one patient with DIP in Beijing Hospital following review of the literatures.Results The patient was a 55-year-old male presented with cough,shortness of breath and hypoxemia.Previous history of smoking and exposure to a variety of metals.ACT scan of the chest revealed diffuse ground-glass densities.A diagnosis of desquamative interstitial pneumonia (DIP) was confiraed by a thoracoscopic open lung biopsy.After treatment with hormone,the condition improved.A total of 66 cases were included in this study,the smoking groupof 20 cases,non-smokers of 46 cases.The smoking group was older than the non-smoking group [(47.5 ± 12.1) years vs (26.1 ± 22.5) years];the present of chest pain,shortness of breath (4/20 vs 0/46;8/20 vs 3/46) was higher in smoking group than in non-smoking group,and ground glass opacity in the chest image(15/20 vs 20/46) was also higher in smoking group than in non-smoking group.Pulmonary function showed more diffuse dysfunction(12/14 vs 6/13) in smoking group than in non-smoking group.Conclusions As a rare disorder,DIP is associated with current or former cigarette smoking and many other risk factors.The clinical presents of non-smoker is atypical.DIP is curable to glucocorticoid and has a good prognosis.There is a possible of recurrence.